Heart to Heart with Anna

Advancements in Understanding the Liver in Fontan Patients Part 1

January 24, 2017 Anna Jaworski
Advancements in Understanding the Liver in Fontan Patients Part 1
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Heart to Heart with Anna
Advancements in Understanding the Liver in Fontan Patients Part 1
Jan 24, 2017
Anna Jaworski

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The Fontan Procedure has been used since the 1970s to treat people with a single ventricle physiology -- also known as a univentricular heart. It has also been used in the last few decades to treat hypoplastic left heart syndrome (HLHS) or hypoplastic right heart syndrome (HRHS). Now there are enough long-term survivors of the Fontan Procedure for the medical establishment to know what kinds of consequences having such a radical physiology poses. When the heart's sole function is to pump blood to the body, and it travels passively to the lungs for oxygen, how does that anatomy affect other organs, specifically the liver?Dr. Fred Wu of Boston Children's Hospital is an expert in working with adults with congenital heart defects with liver involvement. He explains what about the Fontan heart makes involvement with other organs something to be expected and prepared for. He will explain what kind of consequences are commonplace when dealing with a Fontan heart and what kind of monitoring should be done to help patients live healthy, happy lives.

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The Fontan Procedure has been used since the 1970s to treat people with a single ventricle physiology -- also known as a univentricular heart. It has also been used in the last few decades to treat hypoplastic left heart syndrome (HLHS) or hypoplastic right heart syndrome (HRHS). Now there are enough long-term survivors of the Fontan Procedure for the medical establishment to know what kinds of consequences having such a radical physiology poses. When the heart's sole function is to pump blood to the body, and it travels passively to the lungs for oxygen, how does that anatomy affect other organs, specifically the liver?Dr. Fred Wu of Boston Children's Hospital is an expert in working with adults with congenital heart defects with liver involvement. He explains what about the Fontan heart makes involvement with other organs something to be expected and prepared for. He will explain what kind of consequences are commonplace when dealing with a Fontan heart and what kind of monitoring should be done to help patients live healthy, happy lives.

We appreciate it when people support Hearts Unite the Globe podcasts. Thank you to our newest supporters -- Annie Ulchak (Patreon) and Judy Miller (Buzzsprout)!

Support the Show.

Anna's Buzzsprout Affiliate Link

Baby Blue Sound Collective

Social Media Pages:

Apple Podcasts
Facebook
Instagram
MeWe
Twitter
YouTube
Website

spk_0:   0:03
Welcome

spk_1:   0:06
to Heart to Heart with Anna, featuring your host Anna Dworsky. Our program is a program designed to empower the CH D or congenital heart defect community. Our program may also help families who have Children who are chronically ill by bringing information and encouragement to you in order to become an advocate for your community. Now here is an ID Dworsky

spk_2:   0:29
looking to heart to heart. With Anna. I am, and it's your speed and the host of heart to heart. With Hannah, we're in Season nine, and the deepness season is advancements. Congenital heart disease. Our show today is advancements in understanding the liver and Fontanne patients, and our guest is Dr

spk_3:   0:45
Fred Wu. Doctor Boo is board certified in pediatrics, internal medicine and cardiovascular disease. After receiving his doctor of medicine degree from the University of Michigan Medical School in Ann Arbor, he completed a combined residency program in pediatrics and internal medicine at the University of Minnesota Medical Center and a fellowship in cardiovascular disease, also at the University of Minnesota. In 2007 he completed an advanced fellowship in adult congenital heart disease in pulmonary hypertension at Harvard Medical School and stayed on to become a faculty member of the Boston Adult Congenital Heart and Pulmonary Hypertension Program. Dr. Wu has received a Sanofi Aventis Fellow Travel Award for Research, a teaching award from the Harvard Medical School Academy Center for Teaching and Learning Excellence and Grant Funding through Boston Children's Hospitals. Innovation and Digital Health Accelerator for research into innovative approaches to the management of patients with single ventricle physiology. Welcome to Heart to Heart with Anna Dr Bloom

spk_4:   1:48
Thanks. And I'm really excited to chat with you today.

spk_3:   1:50
Well, I'm excited to have you on the program, so let's start by having you explain Wife Bond Tam patients are at risk for developing liver issues.

spk_4:   2:00
Sure, so as many of your listeners will know, the Fontana operation is done in patients who only have one ventricle that's developed enough to support the circulation. So because that one ventricle is needed to pump the blood to the body and to supply all of the vital organs, the surgeons take the two main veins that carry all of the used blood back to the heart to be pumped through the lungs, and they attach those directly to the pulmonary arteries so that the used blood flows into the lungs without going through a pumping chamber first. So because of that arrangement, the blood pressure in the veins after Fontana operation is much higher than is normal in somebody with two functional ventricles. So because the liver is the last organ that most of your blood passes through before it returns to the heart and because the liver is sort of a spongy organ, the increase in venous blood pressure causes delivered to get congested. And over many years that congestion causes scarring or fibrosis in the liver. When you only have one ventricle instead of to, it creates some limitation to the amount of blood that the heart can circulate. And in situations where your body has compromised circulation, it often sacrifices blood flow to the gut in the liver in favor of the other organs. So given those two factors, it's not hard to see why liver diseases such a common problem in the Fontane circulation compared to other types of heart disease.

spk_3:   3:09
Wow, that's the most comprehensive explanation I've ever heard of why they could have a problem. So it would seem to me that universally, people with a single ventricle heart who have had a Fonte an operation will suffer from some kind of liver disease.

spk_4:   3:25
Yeah, I think that's true. And that's pretty much born out of most of the research studies that we've done. Most of the studies have shown that the vast majority of patients, if not all of them, have some degree of liver injury. When we do studies like CT scanning or liver biopsy,

spk_3:   3:39
well, that's kind of scary when you're told that your child is born with hypoplastic left heart syndrome. Like I was, we were told about the three surgeries that he would probably need. But nobody said anything about the fact that those surgeries which saved his life, might also cause liver problems.

spk_4:   3:57
Yeah, you know what I do for a living, which is taking care of adults with congenital heart disease, is really the result of a lot of success is in the field of caring for people with congenital heart disease. So as you know, the Fontana operation was something that wasn't done until the seventies. So we really don't have a lot of long term experience with people who have the Fontaine an operation, so we're really learning more and more as patients who've had the Fontana operation get older. So as you know, there really weren't a lot of people with complex heart disease like single ventricle physiology that we're living to adulthood back 50 years ago. But now we have a lot of Fontane patients who are in their forties and fifties, people that are raising families of their own. And having gone through 30 to 40 years with the Fontane circulation, we're just now learning the sorts of things that can develop in other organs and people who've had the Fontana operation. So a lot of this is still a learning process for us and without having that experience, we don't really know how to counsel people on what's gonna happen to these patients in the very long term.

spk_3:   5:00
Well, that's good to know that it's not like they were trying to withhold information from the horror for many of my friends, but just that it wasn't known, and it's exciting to me to live in a day and age where I can see so many long term survivors. It must be exciting for you, too.

spk_4:   5:16
It is, and that's really what makes our job very rewarding. You know, again, a lot of these patients come to us with stories like you told me earlier, where their parents were told that they should just take their child home and love them until they died. Or in some cases, you know, patients were told that they weren't allowed to exercise or participate in gym class with other kids. And really, what we're finding now is that even with complex heart disease, after having gone through an operation like the Fontane, most of these kids really live very normal lives and will grow up to have fairly normalized as adults as well. We just have to be aware of the possible complications and maintain an appropriate level of surveillance to look for complications that know are likely to happen,

spk_3:   5:57
right? Right. I have had some friends whose Children have developed protein losing an theropod e or a P L E. Is this a result of liver damage?

spk_4:   6:06
Yeah, that's a great question, and it's one that I hear a lot. There's actually probably multiple processes that contribute to the development of protein using enter apathy after the Fontana operation, but it probably isn't a direct consequence of the liver damage. Instead, they probably have some common mechanisms. Soapy Ellie, as you know, is a condition where protein rich fluid leaks into the gut and that can cause low levels of protein in the blood. It can cause swelling of the legs or other soft tissues, and it can cause diarrhea and other G I symptoms. The high vain pressures and the compromised blood flow that also cause injury to the liver probably play an important role in the development of P l E. And there's also some evidence for an inflammatory component, which may or may not contribute to the liver disease as well. So most of the current treatments for PL E are focused on reducing the pressure in the Fontane system, improving the circulation of the heart and then reducing inflammation as well.

spk_3:   6:58
Well, that's really good to know, because if I'm not mistaken, there are also people who don't have a fonte of physiology who can develop Healy. In fact, I have a friend whose son passed away a very young age. I think it was 10 or 11 years old, and they were afraid that it was the kind of heart defect he had had caused the P l E. And so they ended up doing a heart transplant. But sadly, even though he got a new heart, he still had the P l E and ended up passing away because of it.

spk_4:   7:28
Yeah, Appeal Lee is something that has been described in people with inflammatory bowel diseases like Crone's disease, so there are multiple things that can cause the same type of problems. So anything that causes leaking nous of the gut wall, which typically includes some kind of inflammatory process, can cause the blood vessels to get leaky and allow protein to leak out into that space. In the case of people with heart disease, not just Fontane circulation but any type of heart failure, really, the increased pressure in the veins also sort of forces fluid through the wall of the vessels that contributes to the leaking this of the gut as well. So just because somebody has pl e certainly doesn't mean that they have congenital heart disease. Usually, if people are developing purely because of other causes, there's going to be other symptoms that will point the doctor in the direction of what the underlying ideology really is. Fortunately, in the case of congenital heart disease, in most cases, when people do get P l E, it's already known that they have congenital heart disease. Generally, they're gonna have other symptoms of heart failure as well, such as fluid retention for shortness of breath and things like that. So P L. E is rarely the presenting cause for congenital heart disease. And, like you say, there are lots of different things that can cause P. Elia's well,

spk_3:   8:39
well, what are the signs of symptoms that patient may exhibit to alert a parent or the patients doctors that there may be some liver problems occurring?

spk_4:   8:50
Well, that's the difficulty of managing the liver and Fontanne circulation generally when we as doctors or the way public think of people with liver disease such a cirrhosis. We usually picture someone who's had alcoholic cirrhosis or hepatitis, and we think of somebody who has yellow skin, which we call John dis or yellow eyes, which we call victory ists or a swollen abdomen because of fluid buildup, Parasite ese. So while cardiologists are finding that most if not all, people with Fontane circulation have evidence of liver disease, when we do the blood testing or liver imaging. Most of these patients have feel, if any, symptoms. So that's why the onus is really on the primary care doctors and the cardiologists and the liver specialists were taking care of these people to monitor for signs of liver disease because they almost never come to you and present with those symptoms themselves.

spk_3:   9:37
That's really helpful to know. And it's also really scary because there aren't any huge red flags that are alerting people that they probably need to be tested.

spk_4:   9:48
That's right, And I do have to point out that many of the patient's appearance that I talked thio often come because they've heard. Or they've read about cirrhosis and liver disease and Fontanne circulation, and sometimes they're quite alarmed by it. And what I always tell patients is that what we call cirrhosis or liver disease in the Fontane population is probably a different animal than the cirrhosis that we see an alcohol use and hepatitis. So again, with alcohol and hepatitis, we often see people presenting with these signs of liver failure, which include varicose veins in the esophagus or the john disorder societies that we talked about. But in Fontana, patients those symptoms air very uncommon. Generally, patients have other symptoms that are related to the heart disease, but few of any of being better directly related to the liver. And part of the difficulty to is that there is some overlap between symptoms of liver failure and heart failure. So in both heart failure and liver failure, you can get the buildup of fluid in the belly that we call a societies, and you can get swelling in the legs that we call a Dema. And sometimes it can be hard to tease out how much of that is because of their heart disease and how much is because of their liver disease. And the other thing, too, is that when people think about liver disease or cirrhosis in adults who are getting cirrhosis because of other problems, often times there's a very poor prognosis, whereas in the Fontana population, even though we tend to see liver involvement at a very early age, in many cases it doesn't necessarily impact the long term outcomes of these patients, so they don't necessarily develop the morbidity, and it doesn't necessarily contribute to their mortality. In the long term, it does have some very important ramifications as far as how we treat these patients and what their treatment options are in the future. And so that's why it's important for us to keep an eye on it and make sure that it doesn't progress to a point where it does become symptomatic. But again, it's certainly not something that should be alarming to patients. It's something that people should be aware of, and they should make sure that their doctor knows that they need to look out for these sorts of things and to monitor their liver health.

spk_3:   11:41
Well, that just made me feel a 1,000,000 times better.

spk_4:   11:46
That's good. I'm glad to hear that.

spk_3:   11:47
Well, I have a son was a single ventricle heart, and there hasn't been much done regarding his liver, aside from his cardiologists knowing that it's

spk_5:   11:57
not exactly like

spk_3:   11:58
other people's livers who don't have control heart disease. But he hasn't had some of the testing

spk_2:   12:03
that I was afraid maybe he should have had already, so you make me feel a lot better. He doesn't have any of those symptoms. Like you said, he does have a

spk_5:   12:19
hi I'm John Montas of NBC's hit a Capella Show the single and a cappella music. It takes a team to create a sound that many will enjoy. Just like it'll take a team to help my good friend Miles Sweitzer, an H L. H s survivor. Let's help Miles fulfill his dream and make a big enough sound to bring awareness to congenital heart disease. Please visit him at Go Fund me dot com backwards slash the miles project Miles with the Y Again, that's go fund me dot com The Miles Project This is for miles.

spk_2:   12:49
When I saw somebody of the C H Street groups growing, I found family just ready to join May. Anyone who is a member of the adult congenital heart defect community can be a guest on our show way. Have a great year plan that we look forward to sharing other interesting topics. Heart to heart. With Colin David serving a CHD community Wednesday's at Noon Eastern.

spk_0:   13:23
You

spk_1:   13:24
are listening to heart to heart with Anna. If you have a question or comment that you would like a dress on our show, please send an email to Anna Gorski at Anna at heart to heart with anna dot com. That's Ana at heart to heart with anna dot com. Now back to heart to heart with Anna.

spk_2:   13:44
So, Dr Boo, What exactly is liver surveillance

spk_4:   13:48
by liver surveillance? We really just me periodic evaluation of liver health, as opposed to waiting until somebody shows up with obvious liver problems.

spk_3:   13:58
And how would they have their liver assessed?

spk_4:   14:01
Well, that's a good question. So in general, there's consensus among congenital heart disease doctors that liver surveillance should be performed as a matter of routine. But how to do it and went to start doing it isn't entirely clear. Certainly, at every clinic visit, the doctor should be asking about symptoms such as abdominal distension, yellow skin, yellow eyes or any history of coughing or vomiting up blood. Those air obviously pretty significant things that most patients would bring to you if it happened anyway as part of the physical exam. In addition to listening to your heart, the doctor should be examining the belly to see if the liver's enlarged or tender, or if the belly is distended and full of fluid. Blood tests are relatively inexpensive and easy to dio, but for the most part they tend to remain normal even when imaging or liver biopsy shows significant fibrosis in the liver. So even though blood tests are an important part of the surveillance in and of themselves, they're probably not enough for screening for liver health. As faras imaging studies. The main modalities that we use our ultrasound, C T and M R I. Most doctors like to do ultrasound first because it's readily available because it's relatively inexpensive and because it doesn't use any radiation. However, it's relatively insensitive and sometimes misses things like Nah, Jules, especially when there's a background of diffuse liver disease, which is typically what we see in people who've had Fontane Operation. CT and Emory give us a very detailed picture of the liver, and they're probably the best way to monitor the liver, especially in Fontane patients. We generally prefer Emory because it doesn't involve any radiation. So for patients that are needed to get Mariah on a regular basis, you don't get that constant exposure to radiation that can cause problems in the long term. But a lot of patients who have pacemakers or defibrillators can't go into the Emory scanner. So for those individuals, we would tend to use CT scanning instead now, both CT and Emery involved different types of ivy contrast, and they give us more detailed information. But in both cases, the contrast might not be suitable if the patient has kidney disease. So most experts advocate baseline liver testing, using blood tests and liver imaging, starting at about five years after the Fontanez completed. And although individual providers may choose to start a little earlier a little bit later, we encourage doing the test annually and imaging every 3 to 5 years by 15 years after the Fontana, we feel that the patients are probably higher risk. So at that point the liver has been subject to many more years of high Venus pressures, and we recommend more frequent imaging, probably every 2 to 3 years.

spk_3:   16:33
That's the most specific answer I've heard regarding that question that I just asked you, so that was really, really helpful to me now. My son had his spon tan when he was an infant. He was not even a year old, and so he's almost 22 years post Fontane. But he had to have a flinty and revision. Does that play into the years of to a calculator? Do you start from that first Fontane.

spk_4:   17:02
What was your son's initial Fontane in atrial pulmonary Fontanne? Do you know

spk_3:   17:06
he had the Inter Cardiac? He had a ministry of Front E N

spk_4:   17:09
I C. Yeah, Some people undergo Fontane conversion or Fontane revision because there may be areas that become narrow after the initial Fontanez on or because they had older self on tan and we want to update it to a new herself on 10 that may have fewer complications in certain respects. Generally, once the Fontana is done, regardless of the type of Fontane that it is, then the liver is already exposed to those higher venous pressure's. So even if somebody goes through a second Fontana operation later on, their liver has already been exposed to so many years of high pressures and the injury to the liver is probably already started. Most of the time we do. A Fontane Conversion or Fontane revision were actually improving the motive, ma'am. It's so if anything that actually takes some of the stress off of the liver. But regardless of thief Fontane revision are Fontane conversion that we d'oh In the end, as long as you have a Fontane circulation they're still high. Venous Pressure's and deliver does still get exposed to that, and there is still ongoing liver injury.

spk_3:   18:10
Well, that's exactly what I would expect. I've been doing a lot of reading in preparation for our program today, and I write about blood tests and M ars and ultrasounds. But I also read about something called an Alfa Feed a protein test. Can you tell us about that?

spk_4:   18:26
Yeah, The F feet of protein is not a cardiac test. It's It's a tumor marker for liver cancer. And most commonly, it's ordered by liver doctors who are taking care of people with cirrhosis because traditionally it has been one of the ways that they used to screen people for development of liver cancer. Now, in most people, liver cancer is a very rare phenomenon, and it's almost entirely restricted to people who have cirrhosis because of alcohol exposure or hepatitis C or people that have a hepatitis B infection. Because the hepatitis B virus itself is a carcinogen in people with Fontane circulation, we have seen some cases of liver cancer developing, and we feel that it's probably tied to the severity of liver disease, so it's something that most of us don't check routinely. And patients who've had a Fontana operation until they have been at least 15 years out from Fontane completion that will vary from center to center. Generally, I think well, we like to do at Boston Children's Hospital is when we do imaging of the liver. Once we see evidence of more advanced liver disease, then we'll refer to a liver doctor for regular imaging and Alfa feeder protein to screen for the development of liver cancer what they call a paddle cellular carcinoma.

spk_3:   19:38
This is a lot to take in.

spk_4:   19:40
Yeah, it's certainly something that most patients and families find a little bit daunting when we first bring it up. I think the words cirrhosis and cancer and patio cellular carcinoma are certainly very scary things. But again, I want to emphasize that patio Celia carcinoma is a relatively uncommon thing. So although it's been reported in the literature, most centers probably only have had 2 to 3 cases of paddle, so a carcinoma out of a couple of 100 or more Fontane patients that they're following. So it really is a small minority where this develops, but just like any other cancer you want to screen and try to pick these up at a relatively early stage when we still have more treatment options. And the potential for sure is highest in a padded cell carcinoma. Generally, when you see it, just cutting out the cancer is not a good long term cure, because what it tells us is that the underlying liver prank a month underlying liver tissue is diseased enough that the patient is at risk of a patio cellular carcinoma. So what we learned from people who have cirrhosis because of alcohol or hepatitis is if you cut out that pad a cellular carcinoma if you cut out the liver cancer, the likelihood of another liver cancer developing in the next five years is quite high. And that probably holds true for the Fontane circulation, too. If you develop the liver cancer once, you're probably at higher risk of developing it again. So usually when we find somebody who has a liver cancer, we may do surgery, or we may do an ablation to get rid of the tumor at that time. But that changes the long term course of treatment for the patient because ultimately, what we want to do from that point on is usually to d'oh ah, heart, liver transplant and basically get rid of the disease liver so that they don't have that likelihood of getting another liver cancer. Now, one thing that we're still trying to figure out is once you fixed the heart. So if you're to do, ah, heart transplant, but you left deliver alone. We don't know if taking away that high venous pressure and the compromise cardiac output will allow the liver to heal over time. In general, when people have cirrhosis or fibrosis of liver, we presume that the fibrosis is permanent. It's not reversible. But there have been some cases more recently of other kinds of liver disease. Where once people get rid of the underlying problem, whether it's inflammation or infection or what not that the liver fibrosis will actually seem to improve. So there may be some reverse ability there that we weren't aware of before. So that's obviously a very important point because if it turns out that doing a heart transplant will allow the liver to heal, and for some of that fibrosis to reverse, then there would be less of an impetus to do a heart and liver transplant. But right now, most people would say that once a Fontane patient gets liver cancer, the best treatment is to get both a heart and a liver transplant at the same time. I know that's a lot of information.

spk_3:   22:36
It's a lot of information is also daunting because just thinking about getting one organ transplant it is scary enough. But the thought of having to wait for two organs and I imagine you would want to do them both at the same time, so you'd have to find a donor that's suitable. I mean, what is the likelihood that you can get a liver in the heart at the same time?

spk_4:   22:59
I think that's a very good question. And obviously having any kind of transplant is a stressful enough thing to have to go through with thinking about getting a multiple organ transplant, I think is even more stressful for the patient. The good news is that in the limited research that we have when we do a combined heart and liver transplant, the outcomes for the patients tend to be about as good as doing a heart transplant alone. So heart, liver transplant is not something that we tend to do a lot off if you look back in the Yunos database, so you nose is United Network of organ Sharing. So the United Network of organ sharing is what regulates the allocation of organs in the United States, and they do keep very good records. According to their records, Over the past five years or so, there have been 3 to 4 combined heart liver transplant specifically for congenital heart disease each year. So it's not a lot.

spk_3:   23:53
Wow. Yeah, that's really, really small,

spk_4:   23:55
right? And most of those happen in a relatively small handful of centers because you don't want a center that doesn't do a lot of combined transplants to do them because they're outcomes aren't going to be a cz good. So centers like the Mayo Clinic or the University of Pennsylvania in Philadelphia tend to do a lot more than most centers, and their experience generally shows that outcomes tend to be about is good for the combined transplants, as people were getting heart transplant on Lee. The downside is, you point out, is that when you have to wait for two organs, it does tend to make the time on the wait list a little bit longer. And there's also ah, increased chance that you'll wait on the list and never get organs before. Something bad happens. Like if the patient, God forbid, should pass away,

spk_3:   24:39
right, right? Or if one of the other organs should become compromised because you're waiting

spk_4:   24:45
right? And that's actually something that we have had problems with in the past, and that sort of changed how we approach patients now. So 10 years ago, you know, a lot of the patients that we would send to the transplant team for a consideration for transplant would be refused transplant because they had too much liver disease or because they had too much kidney disease. So I think we've been a lot more proactive with what we've learned to monitor for kidney health and liver health and to try to refer those patients to the heart transplant people before they have other organ disease that would make them a poor candidate for transplant.

spk_3:   25:18
Well, that's good to know, and I think that's one of

spk_2:   25:20
the most important reasons have this.

spk_4:   25:23
Oh, absolutely, yeah, that's one of the main

spk_1:   25:25
reasons why we do it you don't want to wait until the patient is too far gone before you send them. Because by that point, obviously there right,

spk_0:   25:35
the most common here is why

spk_2:   25:39
she always needed a lot of attention. She had strokes, even

spk_0:   25:43
though it's a national inclination to withdraw from the CHD community. I think being a part of it,

spk_5:   25:50
help me be part of the solution.

spk_0:   25:53
Heart to heart With Michael Please join us every Thursday at noon. Eastern. I'm Michael even on I'll be your host as we talked with people from around the world who have experienced the most difficult moments you're

spk_1:   26:11
listening to heart to heart with Anna. If you have a question or comment that you would like a dress on our show, please send an email to Anna Gorski at Anna at heart to heart with anna dot com. That's Ana at heart to heart with anna dot com Now back to heart to heart with Anna

spk_2:   26:31
Dr Rioux Fontane Adults differ from center to center or even from doctor to doctor, regarding who is getting screened when they have their baseline information done. Why do you think it very much?

spk_4:   26:45
Well, there's still a lot that we have to learn about liver health and Fontanne adults. As a result, it's been very difficult to issue any guidelines to the congenital heart disease community that are backed up by strong evidence. So I can give you one example. In the past 10 years, practice, even among individuals, is constantly shifting. So when we first realize that people with Fontane circulation we're being diagnosed frequently is having cirrhosis. In Boston, we began performing pretty routine liver biopsies, so any patient that had abnormalities on their blood tests or patients who had evidence of more advanced fibrosis on their CT scans and MRI's would undergo a liver biopsy. And within a couple of years, we were basically saying that almost every single patient had scarring or fibrosis of the liver to some degree. Yet when we went back afterwards and reviewed over 70 liver biopsies that we had done, we weren't able to correlate the degree of scarring with how the patients did in the long term. In other words, the patients that had more severe scarring or more severe fibrosis didn't necessarily get sicker or die sooner than the patients that seem to have milder fibrosis on top of that. We also realized that scarring and the Fontane liver tends to be Apache process, so that called into question how accurate the liver biopsy waas. Depending on where the needle ended up, you could potentially get a piece of relatively healthy tissue. Or you could get into an area that was relatively severely diseased. And we weren't sure that the liver biopsy was actually giving us an accurate assessment of overall that her health. So, as a result, in the course of 10 years, we started doing a lot more liver biopsies. And now we've really backed off on doing liver biopsy with the exception of doing biopsy on suspicious nodule. Is that what you might find on CT or Emery of the liver?

spk_3:   28:28
So it seems to me then, because we didn't talk about the biopsy until just now, it seems to me that the M R I, the CAT scan and the ultrasound I mean you have a whole reservoir of different test at your fingertips, but it doesn't look like there's any definitive test at this point. Is that true?

spk_4:   28:48
That's right, I think. Traditionally, people have considered a liver biopsy to be a gold standard. But at least for people with Fontane associated liver disease, that gold standard is probably in perfect. So we still don't know the optimal way to screen for liver disease. Most of us would say that liver biopsy is not something that should be done routinely, because we really don't know how to interpret the results or what to do with that information. The centers that are continuing to do a liver biopsy generally are doing it as part of a standard regimen. So, for example, of the single ventricle survivorship program at Children's Hospital, Philadelphia, they have been doing liver biopsies on every Fontane patient 10 years Post Fontanne and by doing it in a standardized fashion that does allow us to go back in, evaluate that information, and it takes out any potential for selection by. So we can learn from that. Whether the Fontana liver biopsy is important. If you just do Fontana liver biopsies when you find abnormalities, it's a little bit more difficult to know how to interpret that information. But again, given the shortcomings that we know are inherent to deliver biopsy, that's not something that we recommend as a routine Methodist surveillance. Instead, we focus mostly on doing the blood tests and doing the imaging studies in addition to the usual history and physical.

spk_3:   30:09
Wow, that was a lot of great information that concludes this episode of heart to heart with Anna. Thanks for listening today. Come back next week to hear more from Dr Boob. We're going to talk to Dr Blue about how his research relates to

spk_2:   30:23
palliative care for Children with complex CH D's. You could join us now in our new talk back section immediately following

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the show podcast chat room and my friends, you are not

spk_0:   30:40
thank

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you again for joining us this week. We hope you have been inspired and empowered to become an advocate for the congenital heart defect community. Heart to Heart With Anna with your host, Anna Gorsky can be heard every Tuesday at 12 noon Eastern time. We'll talk again next week

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